TARDBP and amyotrophic lateral sclerosis: In TDP-43-ALS mice, the extent of neuropathology and motor impairment caused by accumulation of TDP-43 aggregates in axons is similar in hemizygous and homozygous TDP-43-ALS mice suggesting that once of certain amount of mutant-TDP-43 accumulates in axons, additional accumulation has no additional effect on axonal transport [214].